Warts this month, steroids in Bell’s palsy, a recap of the year for the new trainees and some more edicts from NICE on what not to do. A couple of links to good CPD opportunities too. Do leave comments below.
More musings from Dr Waterfield this month – this time on paracetamol for immunisation discomfort. Also the 7 important features of a headache y0u must ask about, a link to a very good paediatric emergency medicine site, NICE quality standards in depression, molluscum contagiosum and more musings from me, this time on paediatric phlebotomy. Do leave comments below.
Article by Dr Hajera Sheikh, paediatric registrar
Assessment in Secondary Care
• Lifestyle Assessment
• Menstrual History
• Obstructive Sleep Apnoea: Snoring, difficulty breathing during sleep, morning headaches or fatigue
• Symptoms of co-morbidity including psychological
• Drug use (particularly glucocorticoids and atypical antipsychotics)
• Family history, particularly diabetes <40 yrs, early heart disease <60 yrs
• Height, weight, BMI
• Obesity pattern: generalised, central (greater risk of adverse cardiovascular outcomes), buffalo hump and neck (may be suggestive of Cushing syndrome)
• Blood pressure
• Pubertal assessment
• Acanthosis nigricans (indicative of insulin resistance, first seen round neck and axillae)
• Signs of endocrinopathy
• Dysmorphisms: (Look out for early onset obesity, learning difficulties, deafness, epilepsy, retinitis, dysmorphic features, hypogonadism)
• Thyroid function
• Fasting lipids (total and HDL cholesterol), triglycerides
• Liver function, including ALT
• Fasting glucose and insulin not usually done first line
Refer to Paediatric Obesity/Endocrinology or other specialist service if further investigation is required
• Genetic studies
• Thyroid studies: T3, thyroid antibodies, calcium, phosphate
• Cushing syndrome investigations
• Oral glucose test
• PCOS studies (LH, FSH, adrenal androgens, Sex Hormone Binding Globulin, prolactin, pelvic ultrasound)
• Sleep Study
Dysmorphic and monogenic syndromes associated with obesity:
Main clinical obesity associated syndromes:
• Autosomal dominant
Biemond syndrome (some cases)
• Autosomal recessive
Biemond Syndrome(some cases)
• X-linked inheritance
• Single gene lesions affecting leptin metaboilsm
Congenital leptin deficiency
Leptin receptor mutation
Prohormone convertase 1 mutation
Melanocortin 4 mutation
Clinical features suggesting obesity may be secondary to another condition or syndrome
• Severe unremitting obesity
• Disorders of the eyes
Retinal problems, especially retinitis pigmentosa
Narrow palpebral fissures
Abnormally positioned palpebral fissures
Severe squint (eg Prader-Willi)
• Skeletal abnormalities
• Sensorineural deafness (eg Alstrom syndrome: sensorineural deafness, diabetes mellitus, retinal dystrophy, obesity)
• Microcephaly and/or abnormally shaped skull
• Mental retardation
• Renal abnormalities
• Cardiac abnormalities
With many thanks to Dr Harriet Clompus, paediatric SpR with an interest in community paediatrics for summarising this core-info topic so neatly and usefully.
Emotional Neglect and Abuse
Core-info, a Cardiff university based research group, examines all areas of child abuse by systematically reviewing worldwide literature and producing recommendations based on best evidence. This is a useful resource for paediatricians, general practitioners, health visitors, nurses, social workers, educators. Find all their reviews at www.core-info.cardiff.ac.uk.
Core-info have produced a leaflet in cooperation with National Society of Prevention of Cruelty against Children (NSCPCC) following a review in 2011 of the available literature on emotional neglect and abuse in children less than 6 years old. The leaflet is available at NSCPCC resources at www.nspcc.org.uk/inform. You can also subscribe to CASPAR a news service that signposts you to latest policy, practice and research in child protection.
Definitions of emotional neglect and emotional abuse vary, but all include persistent, harmful interaction with the child by the primary care-giver.
The Core-info/NSPCC leaflet reports one in 10 children in the UK experience severe neglect in childhood. It uses the WHO definitions for emotional neglect and abuse. (World report on violence and health (2002) page 60. Edited by Krug et al)
‘Emotional neglect is the failure of a parent to provide for the emotional development of the child.’
Examples of emotional neglect include:-
– Ignoring the child’s need to interact
– Failing to express positive feelings to the child, showing no emotion in interactions with the child
– Denying the child opportunities for interacting and communicating with peers and adults.
‘Emotional abuse includes failure of a care-giver to provide an adequate and supportive environment and includes acts that have an adverse effect on the emotional health and development of a child. Such acts include restricting a child’s movements, denigration, ridicule, threats and intimidation, discrimination, rejection and other non-physical forms of hostile treatment.’
Examples of emotional abuse include:-
– Persistently telling a child they are worthless or unloved
– Bullying a child or frequently making them frightened
– Persistently ridiculing, making fun of or criticising a child.
The core-info/NSCPCC leaflet categorises behaviour/interactions to be concerned about in three different age groups (it only gives data up to 6 years and on mother (not father or other caregiver) interaction, reflecting data collection in studies reviewed). Attachment to mother is disordered and emotionally neglected children show typical pattern of initially passive and withdrawn and then hostile and disruptive behaviour and developmental delay especially in speech and language.
1) Infant (<12 months old)
- Mother-child interaction: mother insensitive and unresponsive to child’s needs. Rarely speaks to child, describes them as irritating/demanding. Failing to engage emotionally with child during feeds. Child unconcerned when mother leaves and when mother returns, child avoids her or does not go to her for comfort.
- Behaviour: Quiet and passive child. May demonstrate developmental delay within first year, particularly in speech and language (particularly if mother has had depression).
2) Toddlers (1-3 years old)
- Mother-child interaction: More obvious that mother is unresponsive or does not respond appropriately to child (called ‘lacking attunement’). Mother is often critical of child and ignores signals for help. Child is angry and avoidant of their mother.
- Emotionally neglected/abused children grow less passive and more aggressive and hostile, particularly with other children. They show more memory deficits than other children, including physically abused children.
3) Children (3 -6 years)
- Mother-child interaction: Mother offers little or no praise, rarely speaks to the child and shows less positive contact. Mother is unlikely to reach out to the child to relieve distress and the child is unlikely to go to the mother for comfort. Neglectful mothers are more likely to resort to physical punishment than other mothers.
- Emotionally neglected children show more speech and language delay than physically abused children. Girls show more language delay than boys. Their behaviour is often disruptive (rated more disruptive by parents and teachers than physically abused children or controls). They show little creativity in their play, have difficulty interpreting others emotions and have poor interactions with other children. They tend to be less likely to help others or expect help themselves.
Implications for practice:
– All practitioners (gps, paediatricians, nursery nurses and teachers, health visitors etc) need to consider emotional neglect and abuse when assessing a child’s welfare. The longer a child is left in an emotionally neglectful or emotionally abusive environment, the greater the damage. However intensive work with families to increase parental sensitivity to their child’s needs, can lead to improvements in child’s emotional development.
– Important attachment disorders are recognisable in young infants and merit referral to professionals trained in infant mental health (Waltham forest has a Parent Infant Mental Health Service (PIMHS) which accepts referrals related to disordered attachment in children under 3 years. PIMHS works with the mother and child to foster healthier attachment (the earlier in a child’s life this is done, the better the outcome). Any health care professional can refer a family to PIMHS. See paediatric pearls from May 2012 for more information:- www.paediatricpearls.co.uk/…/the-parent-infant-mental-health-service-pimhs
– In older children (>3 years) it can be difficult to know when and where to refer. Emotional neglect and abuse is by definition a persistent behaviour pattern, so cannot be diagnosed on the basis of one short consultation. Concerns about parent-child interaction witnessed in a short consultation in A+E or GP surgery may trigger a health-visitor review to gather information, prior to a possible referral to social services. Information should be sought from all those involved in the child’s care including nursery/school teachers. If concerns around behaviour witnessed in A+E or GP surgery are severe, an immediate referral to social services may be appropriate.
– Professionals should be able to recognise speech and language delay and refer appropriately. See paediatric pearls from April 2012 www.paediatricpearls.co.uk/…/stages-of-normal-speech-development/. Many of the features found in emotionally neglected and abused children may also be observed in those with Autistic Spectrum Disorder (ASD) or Attention Deficit and Hyperactivity Disorder (ADHD). If a child is showing language delay and behavioural disruption they should be referred for a formal child development assessment (either in speech and communication clinic (SACC) or child development clinic (CDC) – refer to Wood Street Child Development team in WF)
– Consider risk factors – Core-info’s systematic review did not encompass ‘risk factors’ for emotional neglect and abuse. However it states that ‘many of these children live in homes where certain risk factors are present. Namely – domestic abuse, maternal substance misuse, parental unemployment or mental health issues, an absence of a helpful supportive social network, lack of intimate emotional support and poverty’.
Lots of things to talk about this month. Reminder of what Koplik spots look like, good e-learning on human trafficking, a link to the new primary care guidelines page, night terrors v. nightmares, some good allergy websites and Jess Spedding again on scaphoid injuries. Do leave comments below.
Parasomnias – with thanks to Dr Sophia Datsopoulos
A group of sleep disorders that are paroxysmal, predictable in timing in the sleep cycle and characterized by retrograde amnesia. Polysomnography (type of sleep study in which various parameters are measured in order to rule in or out various sleep disorders), if performed, is abnormal. Diagnosis is based on a thorough history; extensive work-up seldom necessary.
Focus on: ‘Pavor Nocturnus’ or Night Terrors
Children aged three to eight years, M>F. Often family history of night terrors or sleepwalking. Occur approximately 90 minutes into sleep,
during non-REM sleep.
Presentation: Child suddenly sits bolt upright, screams, and is inconsolable for up to 15 minutes, before relaxing and falling back to sleep
with no memory of the event the next morning. Tachycardia, tachypnoea and other signs of autonomic arousal are apparent.
– Reassure families that they have a benign course and are self-limiting
– Advise them not to attempt to wake the child during an episode and that comforting during the episode may delay its recovery
– Explore and alleviate any stress in the child’s environment. Encourage a relaxing bedtime routine
– If frequent and occurring at a specific times every night, behavioural interventions such as scheduled awakenings (see http://www.epic.edu.au/sites/default/files/Sleep/PDFed/Night%20terrors.pdf) may be beneficial
– More severe forms may benefit from treatment with benzodiazepines (e.g., clonazepam) under direction of specialist services.
Main differential: nightmares – these can occur at any age, during the lighter stage of sleep when dreaming, and so tend to be later in the night. Seizures due to temporal lobe epilepsy can appear similar to night terrors but the seizures are usually brief (30 seconds to a few minutes) and are more common in older children and adults.
Comparison: Night Terrors and Nightmares
|Age||3 – 8 years||Any age|
|Occurrence in sleep cycle||NREM||REM|
|Memory for event||No||Yes|
|Exacerbated by stress||Yes||Yes|
REM = rapid eye movement; NREM = non-rapid eye movement.
Next month: Focus on: Somnambulism (sleep waking) and Somniloquy (sleep talking)
With thanks to Dr Nikolina Kyprianou for this article and to Mr Devesh Misra, consultant paediatric urologist at the Royal London Hospital, for checking it.
Hypospadias is a congenital abnormality where the male urethral opening is displaced ventrally. It may be displaced anywhere within the glans, the shaft of the penis, the scrotum, or the perineum. It results from failure of the urethral folds to close during fetal development and it is often associated with abnormal penile curvature (chordee) due to disrupted development of the penile shaft. Males with severe uncorrected forms of hypospadias may have difficulties in controlling their urinary stream which may require urination in a sitting position. They can also have erectile dysfunction and infertility (due to impaired semen delivery).
The location of the urethral meatus classifies the hypospadias and its severity.
3 classes of severity:
- 1st degree: urethral opening within the glans/subcoronal (50% of cases)
- 2nd degree: urethral opening on the penile shaft (20% of cases)
- 3rd degree: urethral opening within the scrotum or perineum (30% of cases)
Hypospadias results due to disruption of the androgenic stimulation that is required for the normal male external genitalia to develop. Environmental factors have been found to be associated with hypospadias suggesting a multifactorial aetiology. These include:
- Advanced maternal age
- Pre-existing maternal diabetes mellitus
- Gestational age before 37 weeks
- History of paternal hypospadias
- Exposure to smoking and pesticides
Hypospadias is one of the most common congenital abnormalities with an incidence from 0.3 to 0.7% in live male births. It is more common in the Caucasian population and in those of Jewish and Italian descent.
The diagnosis is usually made during the newborn examination. Physical findings include:
- The appearance of two urethral openings: the first one will be in the normal position at the end of the glans, which will be a blind ending urethral pit. The second is the abnormally located true urethral meatus.
- Abnormal foreskin due to its incomplete closure around the glans leading to the appearance of a dorsal hooded prepuce.
- Abnormal penile curvature (chordee).
In 6% of cases the foreskin is normal and the urethral abnormality only becomes evident during/after circumcision when the glans of the penis is visualised.
Isolated hypospadias: this is only rarely associated with upper genitourinary tract abnormalities and therefore there is no need to
routinely perform imaging studies of the tract.
Cryptorchidism and hypospadias: these patients have an increased risk of disorders of sex development. Cryptorchidism is present in 10% of
patients with hypospadias and is higher in patients with proximal hypospadias (eg. scrotal or perineal urethral meatus). Proximal hypospadias on its own is also associated with disorders of sex development. These patients should be referred to a specialist so that they can have the following investigations:
- Pelvic USS to evaluate internal genitalia.
- Karyotype analysis.
- Serum electrolytes as a screen for salt-wasting forms of congenital adrenal hyperplasia.
Hypospadias with other organ system anomalies eg. Congenital cardiac disease, imperforate anus, limb malformations, or cleft lip. These patients require renal and bladder USS because they are at risk of upper tract abnormalities. They should be referred to a specialist who will also perform genetic testing.
Parents should be advised not to circumcise their baby in order to preserve the foreskin and optimise later surgical choices. These patients need to be referred to the local paediatric urologist who will determine the severity of the hypospadias and the need and timing for surgical correction.
The goal of surgery is to create a straight penis by repairing any curvature (orthoplasty), to create a urethra with its meatus at the tip of the penis (urethroplasty), to reform the glans into a more natural conical configuration (glansplasty), to achieve cosmetically acceptable penile skin coverage, and to create a normal-appearing scrotum. Surgery is usually performed within the first year of life because early correction is associated with improved wound
healing, reduced rate of complications and improved emotional and psychological result. The repair is usually planned as a single-stage procedure, but in infants with severe hypospadias a two-stage repair may be necessary.
With the use of modern instruments, sutures and antibiotics and by performing the procedure at a younger age, the repair of hypospadias has been successful.
For a useful leaflet for parents please follow link and download the PDF document: http://www.patient.co.uk/health/Hypospadias.htm
Baskin LS, Erol A, Li YW, Cunha GR. Anatomical studies of hypospadias. J Urol 1998; 160:1108.
Baskin LS, Ebbers MB. Hypospadias: anatomy, etiology, and technique. J Pediatr Surg 2006; 41:463.
Carlson WH, Kisely SR, MacLellan DL. Maternal and fetal risk factors associated with severity of hypospadias: a comparison of mild and severe cases. J Pediatr Urol 2009; 5:283.
Brouwers MM, Feitz WF, Roelofs LA, et al. Risk factors for hypospadias. Eur J Pediatr 2007; 166:671.
Take a look at September 2012’s edition of Paediatric Pearls! Safeguarding issues surrounding head and spinal injuries, simple motor tics, chronic fatigue syndrome, the new CATS website and some pointers to gems you might have missed from the last 3 years. Do leave comments.